Severe neuropathy due to inhalant abuse in adolescents from Pretoria

Inhalation of volatile agents, or solvent abuse, is a dangerous pastime practised by many young adolescents in various parts of the world. Benzine, a distillate of petroleum, is a cheap and readily available solvent that is often inhaled or “sniffed” to produce a short-lived feeling of euphoria or disorientation. The aim of this report is to describe four adolescents with severe polyneuropathies secondary to chronic benzine inhalation who were seen at the Steve Biko Academic Hospital in Pretoria’s Neurology Department. Methods and patients: Four adolescent boys aged 15–18 years presented to the Department of Neurology from 2011 to 2013 with progressive weakness and muscle atrophy. Results: On examination all patients showed signs of a severe motor and sensory neuropathy. Two were wheelchair bound at the time of presentation and an initial diagnosis of Guillain-Barré syndrome was considered. Cerebrospinal fluid analysis was normal and electromyography showed severe mixed motor and sensory mainly axonal polyneuropathies in all patients. All investigations for causes of neuropathies were normal, but all patients eventually admitted that they had been abusing benzine by inhaling it for a period of at least six months. The inhalation occurred as a group activity, involving many children. Conclusion: Inhalant abuse appears to be a common practice amongst adolescents from Pretoria. It can lead to a catastrophic polyneuropathy, which should be considered in the differential diagnosis of a young patient presenting with a Guillain-Barré syndrome-type of clinical picture. Awareness amongst schools and drug programmes should be raised to prevent this tragic and highly disabling condition.http://medpharm.tandfonline.com/loi/ojfp20am201

Severe porphyric neuropathy – importance of screening for porphyria in Guillain-Barré syndrome

The hepatic porphyrias are a group of rare metabolic disorders, each of which is associated with a specific enzymatic alteration in the haem biosynthesis pathway. In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered. The development of acute neurovisceral attacks is related to environmental factors, including medications, hormones and diet. A possible manifestation of a severe attack is rapidly progressing quadriparesis, which may mimic Guillain- Barré syndrome. We present four such cases, highlighting that acute porphyria should be considered in the differential diagnosis of Guillain- Barré syndrome. Three patients presented to Steve Biko Academic Hospital, Pretoria, SA, with progressive quadriparesis, and one to a private hospital with acute abdominal pain followed by rapidly progressive quadriparesis. Two patients had started antiretroviral therapy before the development of symptoms, and one had started antituberculosis therapy. All patients had marked weakness with depressed reflexes, and showed varying degrees of confusion. An initial diagnosis of Guillain-Barré syndrome led to administration of intravenous immunoglobulins in two patients. On testing for porphyria, it was found that two patients had AIP and two VP. Electrophysiological investigations revealed severe mainly motor axonal neuropathy in all. Two patients deteriorated to the point of requiring mechanical ventilation, and one of them died due to complications of critical illness. Haemin was administered to three patients, but the process of obtaining this medication was slow, which delayed the recommended early administration. The surviving patients showed minimal recovery and remained severely disabled. Porphyric neuropathy should always be considered as a differential diagnosis in a patient with an acute neuropathy, especially in SA. Absence of abdominal pain does not exclude the possibility of porphyria, and attacks may be precipitated by antiretroviral and antituberculosis medication. The outcome of our patients was not favourable; specifically, obtaining haemin was a challenge in the state hospital setting.http://www.samj.org.zaam2016Chemical PathologyInternal MedicineNeurolog

Severe porphyric neuropathy - importance of screening for porphyria in Guillain-Barré syndrome

The hepatic porphyrias are a group of rare metabolic disorders, each of which is associated with a specific enzymatic alteration in the haem biosynthesis pathway. In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered. The development of acute neurovisceral attacks is related to environmental factors, including medications, hormones and diet. A possible manifestation of a severe attack is rapidly progressing quadriparesis, which may mimic Guillain-Barré syndrome. We present four such cases, highlighting that acute porphyria should be considered in the differential diagnosis of Guillain-Barré syndrome. Three patients presented to Steve Biko Academic Hospital, Pretoria, SA, with progressive quadriparesis, and one to a private hospital with acute abdominal pain followed by rapidly progressive quadriparesis. Two patients had started antiretroviral therapy before the development of symptoms, and one had started antituberculosis therapy. All patients had marked weakness with depressed reflexes, and showed varying degrees of confusion. An initial diagnosis of Guillain-Barré syndrome led to administration of intravenous immunoglobulins in two patients. On testing for porphyria, it was found that two patients had AIP and two VP. Electrophysiological investigations revealed severe mainly motor axonal neuropathy in all. Two patients deteriorated to the point of requiring mechanical ventilation, and one of them died due to complications of critical illness. Haemin was administered to three patients, but the process of obtaining this medication was slow, which delayed the recommended early administration. The surviving patients showed minimal recovery and remained severely disabled. Porphyric neuropathy should always be considered as a differential diagnosis in a patient with an acute neuropathy, especially in SA. Absence of abdominal pain does not exclude the possibility of porphyria, and attacks may be precipitated by antiretroviral and antituberculosis medication. The outcome of our patients was not favourable; specifically, obtaining haemin was a challenge in the state hospital setting